Table 6.

Self-reported impairment of the joints in patients with severe hemophilia




1972

1978

1985

1992

2001
Aged 0-16 y      
    Median joint score (range)*  1 (0-19)   1 (0-25)   1 (0-10)   0 (0-7)   0 (0-33)  
    Severe joint impairment, proportion of patients (%)  7/65 (11)   8/92 (9)   4/111 (4)   1/92 (1)   4/130 (3)  
Aged 17-25 y      
    Median joint score (range)*  5 (0-16)   4 (0-25)   3 (0-19)   3 (0-12)   3 (0-13)  
    Severe joint impairment, proportion of patients (%)  9/39 (23)   12/54 (22)   11/84 (13)   12/64 (19)   8/42 (19)  
Aged 25-40 y      
    Median joint score (range)*  10 (2-13)   9 (0-22)   8 (0-31)   7 (0-28)   5 (0-24)  
    Severe joint impairment, proportion of patients (%)  22/39 (56)   21/69 (30)   41/115 (36)   42/119 (35)   30/89 (34)  
Older than 40 y of age      
    Median joint score (range)*  12 (4-26)   11 (3-41)   12 (0-42)   12 (0-40)   15 (0-48)  
    Severe joint impairment, proportion of patients (%)  11/16 (69)   16/30 (53)   44/74 (60)   63/113 (56)   102/159 (64)  
Overall      
    Median joint score (range)*  5 (0-26)   5 (0-41)   5 (0-42)   6 (0-40)   5 (0-48)  
    Severe joint impairment, proportion of patients (%)
 
49/159 (31)
 
57/245 (23)
 
100/384 (26)
 
118/388 (30)
 
144/420 (34)
 



1972

1978

1985

1992

2001
Aged 0-16 y      
    Median joint score (range)*  1 (0-19)   1 (0-25)   1 (0-10)   0 (0-7)   0 (0-33)  
    Severe joint impairment, proportion of patients (%)  7/65 (11)   8/92 (9)   4/111 (4)   1/92 (1)   4/130 (3)  
Aged 17-25 y      
    Median joint score (range)*  5 (0-16)   4 (0-25)   3 (0-19)   3 (0-12)   3 (0-13)  
    Severe joint impairment, proportion of patients (%)  9/39 (23)   12/54 (22)   11/84 (13)   12/64 (19)   8/42 (19)  
Aged 25-40 y      
    Median joint score (range)*  10 (2-13)   9 (0-22)   8 (0-31)   7 (0-28)   5 (0-24)  
    Severe joint impairment, proportion of patients (%)  22/39 (56)   21/69 (30)   41/115 (36)   42/119 (35)   30/89 (34)  
Older than 40 y of age      
    Median joint score (range)*  12 (4-26)   11 (3-41)   12 (0-42)   12 (0-40)   15 (0-48)  
    Severe joint impairment, proportion of patients (%)  11/16 (69)   16/30 (53)   44/74 (60)   63/113 (56)   102/159 (64)  
Overall      
    Median joint score (range)*  5 (0-26)   5 (0-41)   5 (0-42)   6 (0-40)   5 (0-48)  
    Severe joint impairment, proportion of patients (%)
 
49/159 (31)
 
57/245 (23)
 
100/384 (26)
 
118/388 (30)
 
144/420 (34)
 

Median joint score over 16 joints (min = 0, max = 48) and severe joint impairment in the left and right ankle, elbow, and knee joints.

*

The median joint score was calculated as the median over the sum of the scores of 16 joints, which have been scored as follows: 0 indicates no impairment; 1, some impairment; 2, some impairment with daily problems; and 3, severe impairment with total loss of function.

Severe impairment with total loss of function reported in 1 or more of the 6 main joints.

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